Cases reported "Choristoma"

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1/48. Posterior scleral choristoma in the organoid nevus syndrome (linear nevus sebaceus of Jadassohn).

    PURPOSE: To highlight the association of posterior osseous and/or cartilaginous ocular choristomas with epibulbar choristomas and the nevus sebaceus of Jadassohn. DESIGN: Small case series. PARTICIPANTS: Four patients with the organoid nevus syndrome. methods: Clinical and histopathologic studies in four patients with epibulbar lesions and nevus sebaceus of Jadassohn. MAIN OUTCOME MEASURES: Ophthalmoscopic findings of peripapillary lesions. Computed tomographic and ultrasonographic characteristic of posterior scleral lesions. Ocular histopathologic findings in one globe from one of the study subjects. RESULTS: Three patients had the triad of posterior osseous/cartilaginous ocular choristomas, anterior epibulbar choristomas, and nevus sebaceus of Jadassohn and one patient had anterior epibulbar choristomas and posterior osseous/cartilaginous ocular choristomas. ultrasonography and computed tomography were valuable in detecting scleral ossification or epibulbar cartilage or both. The ophthalmoscopic findings were similar to those of a choroidal osteoma. CONCLUSIONS: The presence of posterior osseous/cartilaginous ocular choristomas in a patient with epilepsy or epibulbar lesions or both suggests the diagnosis of nevus sebaceus of Jadassohn. Osseous/cartilaginous ocular choristomas should be suspected in patients with nevus sebaceus of Jadassohn and peripapillary hypopigmented fundus lesions.
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2/48. Proliferation of eccrine sweat ducts associated with heterotopic neural tissue (nasal glioma).

    The term "nasal glioma" refers to the presence of heterotopic neural tissue, mainly glial in nature, at or near the root of the nose. We describe a case in which all three components of neural tissue, that is, leptomeninges, glia, and neurons, were present, associated with sweat-duct hyperplasia. Proliferation of sweat ducts is a reactive process in some benign and malignant neoplasms, hamartomas, and cysts. This is the first documented case of hyperplasia of eccrine ductal epithelium induced by nasal glioma.
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3/48. Ectopic lacrimal gland cyst of the orbit.

    Lacrimal duct cysts are not common. It is extremely rare when a lacrimal duct cyst and an ectopic lacrimal gland develop in the orbital cavity. A unique case of an ectopic lacrimal gland cyst of the orbit is presented. A 33-year-old man had a palpable mass above the inferior medial orbital rim for nearly two years. An ocular examination was normal except for a movable, firm mass found in the anterior nasal inferior orbit of the right eye. An echogram revealed a homogeneous, hypoechoic cystic mass. Computed tomography of the orbit showed a well-encapsulated lesion in the lower orbit of the right eye near the inferior rectus muscle, without bony erosion. A tense, thin-walled, clear fluid-filled cyst measuring 15 x 12 x 13 mm in size was completely enucleated without rupture by anterior orbitotomy. Pathologic examination disclosed a small nest of normal gland tissue surrounded by a cystic lesion lined with two layers of lacrimal duct epithelium cells. No recurrent signs were noticed during a 12-month period of follow-up.
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4/48. Ectopic adrenals in a sirenomelic fetus.

    A sirenomelic malformation was diagnosed in a fetus from the first pregnancy of a 25-year-old woman, and it was aborted at the 24th week of gestation. The fetus represented a sympus monopus. The head, organs of the neck, the thorax, and the upper part of abdominal cavity were normally formed. The kidneys, the ureters, the urinary bladder and the urethra were not developed. Adrenals of discoid shape and normal size were present in the normal position. The large bowel ended blindly in the sigmoid colon. Both testes were found in their normal position in the pelvis. Two symmetrical structures, resembling ovaries in shape and colour, were found near the testes. Histological examination established that these two organs were accessory (aberrant) adrenals.
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5/48. Unilateral periventricular nodular heterotopia associated with diffuse areas of cerebral functional abnormalities.

    A 17-year-old boy with polymorphic simple and complex partial seizures is described. magnetic resonance imaging revealed a unilateral periventricular nodular heterotopia near the occipital ventricular right horn. Interictal and ictal electroencephalographic recordings showed bilateral specific epileptiform anomalies in the occipital region and asynchronous slow waves in frontal areas. Single photon emission computed tomography documented a reduction in regional cerebral blood flow in an area of the left occipital cortex and a symmetric increase in tracer uptake in the frontal lobes. The neuropsychologic assessment revealed a dysfunction of the frontal associative areas. Data collected led the authors to suspect a more diffuse cortical dysfunction than the nodular heterotopia revealed on magnetic resonance imaging.
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6/48. Potential pitfall in parathyroid sestamibi imaging resulting from a cardiac pacemaker.

    A 47-year-old woman with a history of end-stage renal disease and hyperparathyroidism after total parathyroidectomy had Tc-99m sestamibi imaging to identify possible ectopic parathyroid tissue. This study was prompted by increasing calcium and parathyroid hormone levels after several years of taking calcium supplements necessitated by a surgically induced hypoparathyroid state and end-stage renal disease. The scan showed persistent linear activity in the upper right mediastinum in delayed images, after washout of the thyroid had occurred. Because of the unusual configuration of this finding, investigation of the patient's clinical history and other imaging studies was undertaken. The authors concluded that the unusual mediastinal uptake was not hyperactive parathyroid tissue, but rather was attributed to the presence of central venous pacemaker wires. Thus, this case illustrates a potential pitfall in parathyroid sestamibi imaging, the uptake of which may increase in the presence of a cardiac pacemaker, and it emphasizes the importance of correlative imaging.
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7/48. Ectopic cervical thymic tissue: diagnosis by fine needle aspiration.

    Cervical thymic masses are congenital lesions that result from aberrant thymic migration during embryogenesis. Although most of these masses are asymptomatic, they may cause debilitating symptoms secondary to encroachment on adjacent aerodigestive structures. Preoperative diagnosis of ectopic thymic tissue is rare; most cases are clinically misinterpreted as branchial cleft remnants or cystic hygromas. Definitive diagnosis has relied on histopathologic examination in nearly all reported cases. However, the invasiveness of open incisional or excisional biopsy carries the risk of surgical and anesthetic complications. Inadvertent surgical thymectomy may result in cell-mediated immune deficiencies in infants and young children. The utility of fine needle aspiration is gaining wider acceptance in the diagnostic evaluation of neck masses. We describe an infant with an asymptomatic cervical thymic mass diagnosed by fine needle aspiration.
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8/48. Ectopic gastric mucosa presenting as a polypoid mass within a Meckel's diverticulum.

    A 32-year-old man presented with severe abdominal pain located in the mesogastrium and right hemi-abdomen. A barium transit study showed a tubular structure of 6 cm arising from a bowel loop in the distal ileum, with an intraluminal polypoid mass near the bottom. diagnosis of a benign lesion within a Meckel's diverticulum was made. Anatomopathology confirmed a Meckel's diverticulum and demonstrated that the polypoid mass was caused by an unusual great ectopic island of gastric mucosa.
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9/48. Persistent mullerian duct syndrome with teratoma in an ectopic testis: imaging features.

    The persistent mullerian duct syndrome represents a rare form of male pseudohermaphroditism, secondary to mullerian inhibiting factor (MIF) deficiency. We describe imaging findings in a 30-year-old male (46 XY karyotype) with bilateral cryptorchidism and mullerian duct anomalies (presence of uterus and fallopian tubes). Grade-III teratoma with yolk sac tumour was detected in one of the undescended testis, lying in the pelvic cavity. The other testis was in the inguinal canal. The rest of the wolffian duct structures (e.g. prostate, seminal vesicles) were nearly normal. Very few reports of imaging findings of this entity have been published thus far, probably because of the rarity of entity, incidental detection of most of the cases at surgery and relatively asymptomatic clinical presentation.
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10/48. Complex ocular choristomas in linear nevus sebaceus syndrome: a report of two cases.

    Two cases of linear nevus sebaceus syndrome (LNSS) are described in which ipsilateral facial nevus of Jadassohn was associated with complex ocular choristoma. One patient also had scleral osteomas, a rare occurrence in LNSS.
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